History and Examination

History

An accurate first-hand witness account of an event is crucial. Ask the child / young person (where appropriate) and any witnesses to describe what happened before, during and after the event. With the consent of the patient / carer, phone witnesses who are not present. Record an event's parts in chronological order.

• Age of the child
• Situation
  • What was the child / adolescent doing when the event happened?
  • Possible triggers: exercise, prolonged standing, getting up suddenly, sleep, sleep deprivation, drugs and alcohol
• Warning symptoms
  • Sweating or feeling warm / hot, vision greying out, muffled hearing - vasovagal syncope
  • Rising feeling in tummy, deja vu, metallic taste / smell - temporal lobe seizure
  • Palpitations, chest pain - cardiac syncope
• The event
  • Eyes - open or shut? eyes off to one side?
  • Circulation - pallor? cyanosis? clamminess?
  • Tone - stiff? floppy? unusual posturing?
  • Movements - a few random jerks (can occur with syncope), or sustained rhythmic jerking? thrashing? pelvic thrusting?
  • Tongue biting - side or front of tongue?
• Duration of event
  • Often inaccurately recalled by witnesses.
• Post event
  • Headache
  • Confusion
  • Weakness down one side

Video

Encourage witnesses / carers to video events on their mobile phone (which though counter-intuitive whilst administering first aid is hugely useful)

Past medical history

• Other funny turns, including number and frequency, and whether the nature of the events is the same each time
• Other symptoms at the time e.g. fever, palpitations
• Patient's medical history / family history of cardiac disease, epilepsy or sudden unexpected death in childhood / young adulthood
• Current medication
• Early childhood and developmental history, psychosocial history and educational attainment

Examination

• Vital signs, lying and standing blood pressure (latter if cardiac or vasovagal syncope suspected), heart sounds and signs of heart failure
• Focal neurological deficits, dysmorphic features and birthmarks (pale patches or multiple café-au-lait patches)

ECG

• If available perform a 12-lead ECG for any episode of collapse, blackout or convulsive episode. See investigations section.

If you believe that the event is due to a condition which requires immediate action, use your clinical judgement to determine the urgency of referral.

Refer a child / young person for an emergency paediatric review with the following features:

• A first seizure with accompanying fever, confusion, coma, or persistently abnormal examination after the seizure
• Rapidly recurring convulsive seizures without other accompanying symptoms / sign
• A child who has sustained significant injury either before or as a result of the event.
• Syncope with abnormal vital signs, cardiac examination and / or ECG.

Where no red flags, diagnose the type of funny turn focusing on the key features in the history. Here is a guide to the commoner causes of funny turns.

• Unresponsive with stiffness and jerking
• Isolated Jerks without collapse / unresponsiveness
• Blank spells
• Going stiff
• Episodes from sleep

Any age

• tonic-clonic seizures – lack of colour change, presence of stertor and sustained stiffening followed by rhythmic jerks. Usually less than 3 minutes. May be triggered by onset of sleep, waking, with sleep deprivation or illness. Some will have focal onset e.g. focal motor, deja-vu, metallic taste or smell, rising feeling in tummy

Infants and young children

• breath holding / reflex anoxic seizures – triggered by crying, screaming, minor fall or injury, fright. Children cry then stop crying become apnoeic and blue ('breath-holding') or white ('reflex anoxic seizures'), stiff with posturing and may jerk.
• infant gratification – triggered by boredom this involves stiffness / thrusting movements (often with tightly adducted legs or against the strap of a high chair / car seat), reddening of the face and variable distractibility. May cry at end.
• motor-stereotypies - rhythmic, fixed, predictable movements that occur both in children developing normally, or in those with developmental problems e.g. autism, speech delay. Examples are head banging, hand flapping, rocking, hair twirling.

School age and adolescence

• vasovagal syncope (simple faint) - stiffening and brief, random jerks in 50% young people. Postural triggers, premonitory symptoms and pallor suggest this diagnosis.
• non-epileptic attacks (dissociative seizures) - in young people over 11 years tend to be more than 10 minutes with thrashing / thrusting movements of varying severity, preceded or followed by distress. Eyes closed. No colour change or stertor.

Any age

• hypnogogic jerks – non-epileptic myoclonic jerks as falling asleep. Extremely common and benign
• myoclonic epileptic seizures – lightning fast jerks singly or in runs. Tend to occur with other seizure types e.g. tonic-clonic seizures, commonest as JME (see below) but can occur at any age

Babies

• benign neonatal sleep myoclonus – non-epileptic myoclonic jerks of limbs, often singly but also in runs, as infant goes off to sleep. Baby otherwise very well. Entirely benign, self-limiting and does not need investigation.

School age and adolescene

• tics – recurrent sometimes complex short gestures such as twitching around the eyes / mouth, grimacing, yawning. Tend to vary over time, and vocal tics can occur. Complexity of tics & feeling of compulsion rules out myoclonus
• juvenile myoclonic epilepsy (JME) – morning myoclonic jerks causing spillage of breakfast or make-up with occasional tonic-clonic seizures suggests this common adolescent epilepsy. Made worse by sleep deprivation

Any age

• daydreaming / 'zoning out' – the commonest cause of blank spells. Often seen at school in children with learning difficulties who struggle to keep up. Children become blank and seemingly unresponsive to vocal distraction or touch. 'Just stare'. The length and lack of other features point to this diagnosis.
• epileptic 'absence' seizures – much less common than zoning out. These last only a few seconds and are associated with occurrence during any activity and other stereotyped features such as eyelid flutter, gulping, swallowing, chewing lips etc. Once seen are spotted several times a day. Clues = brief and become very frequent, other features.

School age and adolescence

• non-epileptic attack disorder (NEADs)- a variation on NEADs described above, but just blank for minutes - hours. Patient may describe own events, not always witnessed by others. Other features may be present e.g. eye closure, variable awareness. Clues are length (longer than epileptic absences) and variability of intensity / nature

Any age

• gastro-oesphageal reflux causing pain and stiffness / posturing. Common in infants and in older children with neurodisability and gastro-oesophgeal reflux. Should relate to feed times. Also known as 'Sandiffer' syndrome.
• tonic seizures - unusual in isolation, may represent frontal seizures especially with unusual posturing / complex behaviour e.g. kneeling during attack.

Infants and young children

• infantile spasms - 3-6 months, clusters of 'salaam attacks': eye rolling, stiffening + collapsing foward for 1-3 seconds. Associated with stalling development and irritability. 'Epileptic encephalopathy' - meaning that the epilepsy threatens normal functioning and development - so needs urgent referral.
• infant gratification - see above.

School age and adolescence

• dystonia - commonest at this age although can occur at any age. Clue (compared to seizures) = painful and worse with activity (think of writers' cramp). Other than this & drug reaction (below), dystonia is rare = specialist advice needed.
• drug reaction - commonest = oculogyric crisis. Ask about anti-emetic use.

Any age

• non-REM parasomnias – sleep-related behaviours tend to occur once per night, usually 1-2 hours after falling asleep. Variants are night terrors (usually a young child 'wakes' terrified but is in fact deeply asleep and unresponsive to comforting), sleep talking, sleep walking and rhythmic limb movements.
• nightmares - waking terrified or upset, responsive to comforting, can recall scary dream, which differentiates from night terrors.
• nocturnal seizures – can occur more than once a night, a key to differentiating from night terrors. Referral for video-EEG sometimes needed to secure diagnosis.

Where available

Perform a 12-lead ECG for any blackout or convulsive episode.

Record a 12-lead electrocardiogram (ECG) using automated interpretation. Red flags include any of the following abnormalities reported on the ECG printout:

• conduction abnormality (for example, complete right or left bundle branch block or any degree of heart block)
• evidence of a long or short QT interval. The normal range for QTc is 350 – 450ms. A QTc greater than or equal to 0.47 seconds is suggestive of a long QT syndrome (LQTS), although a QTc above 0.44 seconds may be considered 'borderline'.
• any ST segment or T wave abnormalities.

If a 12-lead ECG with automated interpretation is not available, take a manual 12-lead ECG reading and have this reviewed by a healthcare professional competent in identifying the following abnormalities:

• Long QT (corrected QT greater than 450 ms) and short QT (corrected QT lower than 350 ms) intervals.
• Ventricular pre-excitation (part of Wolff-Parkinson-White syndrome).
• Abnormal T waves

Further tests depend on the clinical diagnosis.

Do not refer for an EEG to 'rule in' or 'rule out' epilepsy.

Benign neonatal myoclonus of sleep – advise on the benign nature of this condition. Babies usually grow out of it and stop worrying their parents by 4 months of age. There are some good YouTube videos of this condition against which parents can check their own baby for reassurance.

'Blue' breath-holding – see supporting information. Anaemia has been associated with breath-holding spells and correction with iron supplementation can be considered. Drug treatment is seldom necessary. The prognosis is excellent. There is no relationship to epilepsy. Addressing parents' stress and educating about continuing to set boundaries around their child is an important aspect of care.

'Pallid' reflex anoxic seizures – see supporting information. Again, the prognosis is good and there is usually no relationship to epilepsy. Treatment is not usually required although Glycopyrrolate has been successfully used in some. Cardiac pacing is used in some persistently frequent cases.

Motor stereotypies – see supporting information. These are very common in young children and should be considered normal behaviour in most cases. They are frequently seen in children with hearing impairment, speech delay or autistic spectrum disorder. However motor stereotypies do not mean that a disorder such as one of these must be present. They are very unlikely to threaten future development or health. No specific treatment is usually needed for young children.

Night terrors – see supporting information. Reassurance about the benign nature of the problem is the mainstay of treatment. Lifting early in sleep (1/2 hour before the predicted night terror) can prevent the onset of deep sleep and therefore the attacks. This is not usually necessary and can, in some, result in night terrors occurring later in the night.

Vasovagal Syncope – see supporting information. Reassurance, education about triggers and ways to avoid syncope – e.g. avoid skipping meals, plenty of fluids in hot weather / with exercise, maintain salt in diet, continue to do exercise, get up slowly out of bed / chairs. If symptoms start sit / crouch down. Education in schools can help to reduce anxiety and reduce sanctioning of activities / trips etc.

Non-epileptic attacks –offer advice and information (see below). Consider CAMHS referral.

First seizure – Refer to paediatrics – urgency depends on other clinical features – see 'referral' section. Pending the paediatric review offer advice on first aid + safety aspects – see below. Further advice and support can be obtained via the Children's Epilepsy Nurse Specialist at 01392-406547.

Referral Criteria

Refer as emergency if:

• See red flags
• A first seizure with accompanying fever, confusion, coma, or persistently abnormal examination after the seizure
• Rapidly recurring convulsive seizures
• A child who has sustained significant injury either before or as a result of the event.
• Infantile spasms (see 'going stiff')
• Syncope with abnormal vital signs, cardiac examination and / or ECG.

Refer as urgent:

• A child with a history suggestive of vasovagal syncope but risk factors exist e.g. family history of sudden unexpected death in young life, where examination & 12-lead ECG are normal. Refer to paediatrician with cardiology expertise. If vital signs / cardiac exam / ECG abnormal make emergency referral
• A child with a history suggestive of one or more unprovoked (epileptic) seizures should be referred to a paediatrician with epilepsy / neurology expertise.

Refer as routine:

• Any child other than the above where you have doubt about the diagnosis.

Do not routinely refer - see management section above

• A baby with benign neonatal myoclonus of sleep
• A toddler with breath-holding attacks or reflex anoxic seizures
• A child with tics or a motor stereotypy
• A child with night terrors
• A child / adolescent with vasovagal syncope and / or pre-syncope
• An adolescent with non-epileptic attacks (dissociative seizures) check GP feelings? AB

Referral Instructions

To refer as emergency:

• Contact the acute paediatrics via the on-call team

To refer to outpatients:

e-Referral selection

• Priority: Urgent/Routine
• Speciality: Children's & Adolescent Services
• Clinic type: Cardiology/Neurology/Other medical
• Service: DRSS-Eastern-Child & Adolescent Services-Devon ICB - 15N

Referral Form

DRSS Referral Form

Blue breath-holding attacks and reflex anoxic seizures - NHS choices
Good advice on both types of episode, distinguishing between the two and promoting self-management as well as 'when to see your GP'

Reflex anoxic seizures - STARS - video + PDF available

Motor stereotypies - Johns Hopkins Medicine (although this is American and talks about research into behavioural management in USA and is geared to children in whom they persist into school age)

Night terrors and nightmares - NHS choices

Vasovagal Syncope - STARS - video & PDF, here called 'reflex syncope'.

• Also links to advice for teachers and school staff

Non-epileptic attacks – supportive information including 'what people should do if I have a seizure' advice for schools at Non_Epileptic Attacks

First seizure – first aid advice depends on the type of seizure.

• For a tonic-clonic seizure PDF on first aid visit
• For simple (alert) or complex (impaired consciousness) focal seizures pick out the relevant section of the Epilepsy Society first aid pages
• Offer safety advice in case of future seizures. In children focus on safe bathing. See Epilepsy Action advice

Evidence

1. NICE Clinical Guideline on Transient Loss of Consciousness (blackouts) in over 16s

2. NICE Clinical Guideline on the Epilepsies in Children and Adults

Pathway Group

This guideline has been signed off on behalf of NHS Devon.

Publication date: 02 December 2016